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Congenital pulmonary-airway malformation: Definition.

Malformation of part of the lung parenchyma, with abnormal direct connections to the normal tracheobronchial tree.

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Congenital pulmonary-airway malformation: Blood supply.

Derived from the pulmonary circulation.

2

Congenital pulmonary-airway malformation, type 0: Gross pathology.

Lung parenchyma appears solid.

3

Congenital pulmonary-airway malformation, type 0: Histology (2).

Disorganized proximal airways; no distal airways.

Intervening tissue consists of connective tissue, thick-walled arteries, and extramedullary hematopoiesis.

4

Congenital pulmonary-airway malformation, type 1: Gross pathology.

Medium and large interconnecting cysts, usually limited to one lobe.

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Congenital pulmonary-airway malformation, type 1: Histology.

Cysts are lined by bronchial epithelium and often contain bands of smooth muscle.

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Congenital pulmonary-airway malformation, type 2: Gross pathology.

Back-to-back cysts, smaller than the cysts of type 1.

7

Congenital pulmonary-airway malformation, type 2: Histology.

Cysts are separated by alveolar ductlike structures, blood vessels, skeletal muscle.

8

Congenital pulmonary-airway malformation, type 2: Associations.

Sirenomelia.

Renal malformations.

Diaphragmatic hernia.

Cardiovascular abnormalities.

9

Congenital pulmonary-airway malformation, type 3: Gross pathology.

Solid mass involving the lobe or the entire lung, with hypoplasia of the other lung.

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Congenital pulmonary-airway malformation, type 3: Histology.

Glandlike structures lined by low cuboidal epithelium.

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Congenital pulmonary-airway malformation, type 3: Associations.

Male sex.

Polyhydramnios.

12

Congenital pulmonary-airway malformation, type 4: Gross pathology.

Peripherally located cysts of variable size.

13

Congenital pulmonary-airway malformation, type 4: Histology.

Cysts can have thick walls and are lined by a single layer of pneumocytes.

Capillary beds beneath the epithelial lining.

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Congenital pulmonary-airway malformation, type 4: IHC.

Pneumocytes express TTF-1 and surfactant proteins A and B.

15

Congenital pulmonary-airway malformation: Limitations on diagnosis.

Diagnosis cannot be made when there is chronic inflammation and fibrosis.

16

Bronchopulmonary sequestration: Definition (2).

Anomalous segment of lung has no connection with the normal tracheobronchial tree.

Blood supply comes from the systemic circulation.

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Bronchopulmonary sequestration: Types (2).

Intralobar.

Extralobar.

18

Bronchopulmonary sequestration, intralobar type: Typical location.

Lower lobe.

19

Bronchopulmonary sequestration, intralobar type: Gross pathology (3).

No pleural covering.

Sharply demarcated from the normal lung tissue.

May have vascular pedicle.

20

Bronchopulmonary sequestration, intralobar type: Histology (3).

Marked chronic inflammation.

Mucus accumulation and microcyst formation.

Dense fibrosis surrounds remnants of bronchioles.

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Bronchopulmonary sequestration, extralobar type: Typical location.

Left side.

22

Bronchopulmonary sequestration, extralobar type: Gross pathology (2).

Covered with visceral pleura.

Separate from normal lung tissue.

23

Bronchopulmonary sequestration, extralobar type: Histology (3).

Enlarged bronchi, bronchioles, alveoli.

No significant inflammation or fibrosis.

Dilated subpleural lymphatics.

24

Bronchopulmonary sequestration, extralobar type: Association.

Congenital pulmonary-airway malformation, type 2, is present in up to half of cases.

25

Bronchogenic cyst: Origin.

Anomalous budding of the tracheobronchial anlage during development.

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Bronchogenic cyst: Most common site.

Mediastinum.

No communication with the tracheobronchial tree.

27

Bronchogenic cyst: Radiography.

May have an air-fluid level.

28

Bronchogenic cyst: Histology (3).

Epithelial lining and wall (smooth muscle, cartilage) resemble those of a normal bronchus.

Squamous metaplasia or chronic inflammation may occur.

No alveolar tissue.

29

Congenital lobar emphysema: Definition.

Hyperinflation of one or more lobes of the lung.